Congenital isolated Iso–Kikuchi syndrome in a newborn

Abstract

Key Clinical MessageClassic CO (also called Iso–Kikuchi syndrome) represents a benign, isolated condition associated with normal patient outcome. Nevertheless, clinical follow-up and/or further clinically-based tests are needed to exclude other nail diseases associated with multisystem pathology; complete family history is also important to determine sporadic or hereditary transmission of such condition.

Iso-Kikuchi Syndrome in A Newborn and His Mother

“…International incidence is about 4.2/100000 births [3]. It was first reported in Japan, and later in other countries such as the USA, England, France, Korea [5], Argentina [1], Brazil [7] and Italy [3,5,8]. It affects primarily the nails of the index fingers, especially the medial aspect of the nail and it can also affect the underlying bone [1][2][3][4]8].…”

“…Years later, in 1980, Baran and Stroud suggested a modification of the criteria: congenital occurrence, unilateral or bilateral index finger involvement, variability in nail appearance, possible hereditary involvement and frequently associated bone abnormalities [1,3,4,7,8,11].…”

Micronychia of the Index Finger

“…Hidrotic ectodermal dysplasia exhibits onychodystrophy, generalized hypotrichosis, palmoplantar keratoderma, and dental anomalies. 7 Nail-patella syndrome presents with hypoplasia of the fingernails and toenails, triangular nail lunulae, absent or hypoplastic patellae, and elbow and iliac horn dysplasia. Poland syndrome is distinguished from COIF by the congenital absence of the pectoralis major muscle on the ipsilateral side of the involved digits.…”

Iso‐Kikuchi syndrome with Y‐shaped bifurcation of the distal phalanx of the index fingers

“…The clinical presentation is characterized by anonychia or nail dysplasia in the form of micronychia, polyonychia (rudimentary splitting), hemi-onychogryphosis, irregular lunula, and nail misalignment [6,7]. Associated systemic disease or malformations have not been described [6][7][8][9]. Prognosis is excellent, with clinical stability and no worsening of symptoms.…”